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- William Kuo, Sang Le, Eugene Kim, and Carl Yuan-Feng Lo.
- From the Department of Anesthesiology, Keck School of Medicine of the University of Southern California, Los Angeles, California.
- A A Pract. 2023 Oct 1; 17 (10): e01723e01723.
AbstractCongenital myasthenic syndromes are rare genetic diseases involving pathologic proteins in the neuromuscular junction. Malignant hyperthermia susceptibility is a genetic disorder involving a hypermetabolic response to volatile anesthetics and depolarizing neuromuscular blocking agents. We present the first reported case of a 3-year-old boy with both congenital myasthenic syndrome and malignant hyperthermia susceptibility, resulting from a mutation in the ryanodine receptor type 1 gene, who underwent an adenotonsillectomy for severe obstructive sleep apnea. We discuss the anesthetic challenges in navigating these 3 comorbidities in the setting of airway surgery.Copyright © 2023 International Anesthesia Research Society.
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